Persistent Leucine Sensitivity Following Partial Pancreatectomy and Diazoxide Treatment: Therapeutic Response

Hyperinsulinemic hypoglycemia secondary to nesidioblastosis persisted in a 4-year-old girl despite a 95% pancreatectomy and high-dose diazoxide treatment. Current literature suggests that dietary treatment, specifically protein restriction, of patients who are receiving diazoxide is ineffective and that further surgical pancreatectomy is required. However, in this patient, marked leucine sensitivity was demonstrated and a subsequent reduction in leucine intake eliminated hypoglycemia and the need for total or nearly total pancreatectomy. Therefore, dietary alteration should be considered in some patients with hyperinsulinism as an adjunct to other medical treatment.